Treatment response of CNS high-grade neuroepithelial tumors with MN1 alteration

Pediatr Blood Cancer. 2020 Dec;67(12):e28627. doi: 10.1002/pbc.28627. Epub 2020 Sep 22.

Abstract

Central nervous system high-grade neuroepithelial tumor with MN1 alteration (CNS HGNET-MN1) is a rare recently described entity. Fourteen CNS HGNET-MN1 patients were identified using genome-wide methylation arrays/RT-PCR across seven institutions. All patients had surgery (gross total resection: 10; subtotal resection: four) as initial management followed by observation alone in three patients, followed by radiotherapy in eight patients (focal: five; craniospinal: two; CyberKnife: one) and systemic chemotherapy in three patients. Seven patients relapsed; five local and two metastatic, despite adjuvant radiotherapy, of which three died. Treatment of CNS HGNET-MN1 remains a major treatment challenge despite aggressive surgical resections and upfront radiotherapy, warranting new approaches to this rare malignancy.

Keywords: CNS HGNET-MN1; MN1; astroblastoma; ependymoma; neuroepithelial tumor.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Central Nervous System Neoplasms / genetics
  • Central Nervous System Neoplasms / pathology*
  • Central Nervous System Neoplasms / therapy
  • Child
  • Child, Preschool
  • Combined Modality Therapy
  • Female
  • Follow-Up Studies
  • Humans
  • Male
  • Mutation*
  • Neoplasms, Neuroepithelial / genetics
  • Neoplasms, Neuroepithelial / pathology*
  • Neoplasms, Neuroepithelial / therapy
  • Prognosis
  • Retrospective Studies
  • Trans-Activators / genetics*
  • Tumor Suppressor Proteins / genetics*
  • Young Adult

Substances

  • MN1 protein, human
  • Trans-Activators
  • Tumor Suppressor Proteins

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